Department of Pediatric Cardiology, Kanuni Sultan Suleyman Training and Research Hospital, İstanbul, Turkey.
Department of Maternal and Fetal Medicine, Kanuni Sultan Suleyman Training and Research Hospital, İstanbul, Turkey.
J Ultrasound Med. 2021 Dec;40(12):2721-2726. doi: 10.1002/jum.15669. Epub 2021 Mar 3.
The aim of this study is to share our experience in the prenatal diagnosis and characteristics of double aortic arc and neonatal consequences.
We retrospectively analyzed 2153 fetal echocardiography reports between 2014 and 2019 years. Records of 14 fetuses with double aortic arc were examined. Prenatal and postnatal medical records, sonographic images, genetic reports, associated cardiac and extracardiac anomalies, and neonatal clinical results of affected fetuses were reviewed retrospectively.
DAA was isolated in 9 of 14 (64.2%) cases, while the other five cases had cardiac or extracardiac accompanying ultrasound findings. Three of cases (21.4%) were associated with other heart pathologies, including ventricular septal defect, double outlet right ventricle, and persistent left superior vena cava. In 10 cases (71.43%), the right aortic arch diameter was dominant. The left aortic arc was dominant in two cases and both arcs were symmetrical in the remaining two cases. 22q11 microdeletion was the only chromosomal abnormality and was detected in two of nine patients who accepted genetic analysis. Intrauterine fetal death occurred in two of the cases. After birth, in 58.3% (7/12) of the live born cases various degrees of symptoms. Surgical repair was performed with the division of the aorta to all symptomatic cases at different times according to severity of the complaints.
Since it can cause severe respiratory distress in the postnatal period and may accompany chromosomal anomalies, it is important to provide antenatal diagnosis of double aortic arc and adequate counseling to the family.
本研究旨在分享我们在产前诊断、双主动脉弓的特征和新生儿结局方面的经验。
我们回顾性分析了 2014 年至 2019 年间的 2153 例胎儿超声心动图报告。检查了 14 例双主动脉弓胎儿的记录。回顾性分析了受影响胎儿的产前和产后病历、超声图像、遗传报告、相关心脏和心脏外异常以及新生儿临床结果。
9 例（64.2%）为孤立性 DAA，5 例有心脏或心脏外超声表现。3 例（21.4%）合并其他心脏病变，包括室间隔缺损、右心室双出口和永存左上腔静脉。10 例（71.43%）右主动脉弓直径占优势，2 例左主动脉弓占优势，2 例两弓对称。22q11 微缺失是唯一的染色体异常，9 例接受遗传分析的患者中有 2 例发现该异常。2 例胎儿宫内死亡。出生后，12 例活产儿中有 7 例（58.3%）出现不同程度的症状。根据症状严重程度，所有有症状的病例均在不同时间行主动脉分割术进行手术修复。
由于双主动脉弓在出生后可引起严重的呼吸困难，并且可能伴有染色体异常，因此对双主动脉弓进行产前诊断并为家庭提供充分的咨询非常重要。
